Management of Von Willebrand Disease with Leukocytosis

Diagnostic Evaluation

A complete VWD workup should include VWF:Ag, VWF:RCo, and the ratio of VWF:RCo/VWF:Ag to properly classify the type of VWD, as recommended by the American College of Medical Genetics and Genomics 1
Sample collection and processing conditions significantly affect test results, so proper handling at room temperature and prompt processing are essential to avoid false results, according to the College of American Pathologists 1

Waldenström's macroglobulinemia can present with leukocytosis and may be associated with acquired von Willebrand disease, as reported by the International Workshop on Waldenström's Macroglobulinemia 2, 3
Myeloproliferative neoplasms (MPNs) like polycythemia vera and essential thrombocythemia can cause leukocytosis and are associated with acquired von Willebrand syndrome, according to the World Health Organization 4
Inflammatory conditions can cause both leukocytosis and elevated VWF levels, potentially masking underlying VWD, as noted by the European Hematology Association 1, 5

Aspirin should be avoided in patients with extreme thrombocytosis and confirmed AvWS due to increased bleeding risk, as recommended by the American Society of Hematology 6
Bortezomib-based therapy is recommended for patients with Waldenström's macroglobulinemia who have high IgM levels, symptomatic hyperviscosity, or acquired VWD, according to the International Workshop on Waldenström's Macroglobulinemia 3, 7
Rituximab-based regimens remain a recommended primary therapy for most patients with Waldenström's macroglobulinemia, as stated by the National Comprehensive Cancer Network 7, 8

Acquired von Willebrand syndrome in MPNs is associated with decreased large von Willebrand factor multimers, which can be corrected with cytoreduction, as reported by the Mayo Clinic 4
Relying solely on FVIII levels for treatment decisions is a common pitfall - VWF activity is the critical parameter, as emphasized by the American College of Medical Genetics and Genomics 1
For women with VWD and leukocytosis planning pregnancy, a multidisciplinary approach with early involvement of high-risk obstetrics is strongly recommended, as advised by the American College of Obstetricians and Gynecologists 6

For surgical patients, monitor VWF:RCo, VWF:Ag, and FVIII levels pre-treatment, post-treatment, and periodically during treatment, as recommended by the Society of Thoracic Surgeons 1
For persistent mucosal bleeding despite adequate FVIII levels, consider adjunctive treatments like tranexamic acid, as suggested by the European Hematology Association 1
In patients with Waldenström's macroglobulinemia, regular monitoring of IgM levels and fundoscopic examination for signs of hyperviscosity are recommended, as stated by the International Workshop on Waldenström's Macroglobulinemia 2